Pheochromocytomas are rare, catecholamine-secreting, adrenal neoplasms. In about 25% of cases they arise in patients with germline mutations. Malignancy occurs in about 10%.
We retrospectively analysed the records of patients with histological diagnosis of pheochromocytoma submitted to adrenal surgery between 19872008 and followed in the Endocrinology department.
Thirteen patients were included. We evaluated age on diagnosis; clinical presentation, urinary concentration of cathecolamine metabolites; imaging at diagnosis; pre-operative management; surgical complications and clinical evolution.
Mean age was 45.2±20.2 years (2077); seven patients (53.8%) were males. Six patients (46.2%) had sustained hypertension, two presented with acute pulmonary oedema. The mean delay of diagnosis after clinical presentation of hypertension was 5.6±6.2 year. Four cases (30.8%) presented as incidentalomas. Mean tumor size was 6.4±3.2 cm (1.413.8); six cases (46%) were on the right adrenal gland and two were bilateral. Mean urinary metanephrines increase was 8.9 times from the reference range; Vanilmandelic acid was normal in three cases. The mean fenoxibenzamine dose used in the pre-operative preparation was 28.3±7.5 mg. The most common surgical complication was hypotension after removal of the tumour. The surgical approach was made by laparotomy in six cases and by laparoscopy in three. Two patients had malignant sporadic pheochromocytoma, one died 9 years after surgery, the other has been followed for 21 years and has received five MIBG treatments for bone metastasis. Four patients were lost to follow-up and eight (61.6%) are in remission for 6.5±5.5 years. Four of them belonged to two different families and had MEN2A. In this cohort, tumour size did not significantly correlate with urinary metanephrines (ro=0.4, P=0.35, Spearman test).
In conclusion, urinary metanephrines were the most useful method in confirming diagnosis. Incidentalomas were a frequent presentation. Establishing the prognosis of these situations remains difficult so close follow-up is required in order to prevent further complications.
25 - 29 Apr 2009
European Society of Endocrinology